A Singaporean Patient Story About Her Rare Disease – Hereditary Angioedema (HAE).

What happened on Sunday 17/01/21

  • swollen neck
  • Swollen foot
  • swollen throat

I acted quite fast. I texted my husband and told him that my throat was swelling. He knows the drill. Anything that attacks the face, throat, or abdomen is an emergency. It has happened before.Upon arrival at the hospital, I was really hungry, knowing that my throat was swelling and because my pain threshold is very high, I could still have brunch, but I only had soft tofu and soup. Lucky I ate something because once I was at A&E, I wouldn’t be allowed to eat or drink.

It was very fast. I was given priority, and I prompted the nurses that I’m having an HAE attack in my throat. Giving me the priority is their standard operating procedure. Everything happened so fast; they took my blood, put an IV plug in me, then I was wheeled to RESUS. In there, I am already strapped with wires, and they scope my throat. Given I am eight weeks pregnant makes this whole situation much more complicated as the doctor needs to figure what to give me or not. A few minutes after the scope, they administered a particular medicine – I can remember exactly what it was, but it was something like hydrocortisone. Soon after, my throat felt so tight it feels like something is pushing me against the wall, and I can’t breathe. Plus, I have sore throat pain.

I was already half awake and groggy. A doctor from the high dependency ward came down to see me and told me they would bring me up to the ward soon and that they are going to put me on close monitoring. Before bringing me up to the ward, they went over the risks of the medicine my doctor suggested they give me, which was C1 Inh Esterase. This treatment is expensive in Singapore – but this is the ‘mother of all’ emergency medicines for HAE and the only treatment available in hospitals for severe attacks in Singapore. I fully understand what is about to happen, but I’m not worried about this medicine’s price at this time.  C1 Inh Esterase is just another rare disease medicine that no one can afford in Singapore. I am only worried about the human growing inside me and myself. I just nodded and said ‘yes’ after hearing the risk and the medical jargon the doctor explained.  I just nodded again and replied, “I understand.”

Because of my pregnancy, I am unable to take the only available preventative treatment in Singapore.  This treatment is an androgen and has many unpleasant side effects, but I use it because I know how expensive the acute treatment is and that it is not always available to everyone.

In the ward, they took my weight for the medicine. I need three boxes of C1 Inh Esterase as the treatment is based on my weight.  Once they administered the medication, the swelling doesn’t go away immediately, and it still takes few hours. But I felt the difference by the hour.

I thank the medical team, the doctors for keeping me sane. They didn’t tell me that my airway was already close 60% – they told my husband. I think if the doctors had said this to me, I would have panicked and cried. I thanked them for my life. I’m thankful that I acted quite fast and went immediately to the hospital, knowing the attack was getting worse.  If I am still my old self in denial, I guess I would only go to the hospital at the very last minute, but by then, I don’t know if I’m going to make it alive or not. I’m really very thankful and blessed for the people around me.

I feel that my story needs to be heard in Singapore and all over the world. Anyone who is just like me, or afraid, you are not alone. We can relate to you. I’m thankful to have come across HAE International and know Fiona Wardman (patient, HAEi Regional Patient Advocate for Asia Pacific), and be part of the HAEi community and the patient lead for my own country Singapore with the help of Fiona. My wish and aims are that patients in Singapore with HAE will accept and have access to modern treatments.

Written by Ms. Nurul Jannah

Ms. Nurul Jannah passed away in May 2022 due to a HAE attack leaving behind her husband and 3 very young children.